Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse cystic lesions of the lung. The present study was designed to evaluate the right ventricular (RV) function in LAM patients via single-beat real-time three-dimensional echocardiography (RT-3DE) and to investigate the factors affecting RV function in LAM patients. According to tricuspid regurgitation velocity (TRV), forty-five female LAM patients [(44.07 ± 10.22) years old] were divided into TRV ≤ 2.8 m/s group (n = 29) and TRV > 2.8 m/s group (n = 16). Relative echocardiography parameters were assessed by conventional transthoracic echocardiography, Doppler tissue imaging (DTI) and RT-3DE, respectively. Pulmonary function tests and the six-minute walk tests (SMWT) were also performed for LAM patients. We found that most of RV functional parameters in LAM patients were worse than that in control patients, although left ventricular dysfunction was not significantly observed. Correlation analysis showed that 3D echocardiographic RV ejection fraction (RVEF) was negatively correlated with pulmonary vascular resistance (PVR), TRV, and the decrease of oxygen saturation (SpO2) post SMWT, and positively correlated with Forced expiratory volume in the first second/forced vital capacity, carbon monoxide diffusion predicted value, SMWT distance, and resting SpO2 in LAM patients. Multivariate stepwise linear regression analysis showed that PVR and SpO2 before SMWT were independent influence factors of RVEF in LAM patients. In this study, we found that RV dysfunction was presented in LAM patients, although left ventricular dysfunction was not significantly obvious. The main influence factors of RVEF were PVR and hypoxia. RT-3DE is a low-cost and noninvasive way to evaluate RV function in LAM patients.
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TomTec Right Ventricular Analysis.
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Funding
This work was funded by Shanghai Three-year Plan of Action for Further Accelerating the Development of Traditional Chinese Medicine (2018-2020) [NO. ZY (2018-2020)-FWTX-1003], Shanghai Comprehensive Hospital Integrated Chinese and Western Medicine Project (ZHYY-ZXYJHZX-1-06) and Shanghai Municipal Education Commission – Gaofeng Clinical Medicine Grant Support (20161402).
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YF, JQ and MZ conceived and designed the study. WH, WY, JG, JW, WW, YL and HZ collected the data and performed analysis. WH and WY drafted manuscript. All authors critically reviewed data analysis and the manuscript. All authors read and approved the final manuscript.
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the Ethics Committee of Ruijin Hospital Affiliated with Shanghai Jiaotong University School of Medicine.
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Hua, W., Yang, W., Gu, J. et al. Risk factors for right ventricular dysfunction in patients with lymphangioleiomyomatosis. Int J Cardiovasc Imaging 37, 439–448 (2021). https://doi.org/10.1007/s10554-020-02009-x
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DOI: https://doi.org/10.1007/s10554-020-02009-x